• Title/Summary/Keyword: 선천성 심장 기형

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Congenital Pulmonary Vein Stenosis with Normal Anatomical Connection -One case report- (정상적인 해부학적 연결을 가진 선천성 폐정맥 협착증 -치험 1예 보고-)

  • 박준석;장윤희;정미진;강이석;전태국
    • Journal of Chest Surgery
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    • v.37 no.4
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    • pp.364-368
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    • 2004
  • Congenital pulmonary vein stenosis is a rare anomaly and related to high mortality due to progressive pulmonary hypertension and heart failure in infancy. Aggressive anti-failure medication and surgical treatment is recommended. Surgical options are balloon dilatation, endovascular stent, pneumonectomy, lung transplantation, patch grafting, and sutureless repair. We report a case of congenital pulmonary vein stenosis with normal anatomical connection successfully treated with sutureless technique and using pulmonary vasodilators, such as Sildenafil, lloprost and iNO postoperatively.

Right Pulmonary Artery Agenesis -A Case Report- (우측 폐동맥 형성부전증 -수술치험 1례-)

  • Sin, Dong-Geun;Kim, Min-Ho;Kim, Gong-Su
    • Journal of Chest Surgery
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    • v.30 no.1
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    • pp.108-111
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    • 1997
  • Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

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Nuss Operation with Simultaneous Intracardiac Repair -A case report- (선천성 심기형에 동반된 누두흉에서 동시에 시행안 심기형 교정 및 Nuss 수술 -1예 보고-)

  • 허재학;장지민;김욱성;장우익;정철현
    • Journal of Chest Surgery
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    • v.37 no.6
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    • pp.536-538
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    • 2004
  • There are still some controversies regarding one stage repair of pectus deformity with congenital heart disease due to probable complications after extensive resection of deformed cartilages. We performed Nuss operation with simul-taneous intracardiac repair in 8 year old patient with pectus excavatum and partial atrioventricular septal defect. The result of operation was satisfactory without prolongation of operation time, bleeding or instability of chest wall.

Congenital Cystic Adenomatoid Malformation (선천성 낭포성 선종양 기형 -1례 보고-)

  • 장기경
    • Journal of Chest Surgery
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    • v.28 no.7
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    • pp.726-730
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    • 1995
  • Congenital cystic adenomatoid malformation[CCAM of the lung is extremely rare. We have experienced an unusual case of congenital cystic adenomatoid malformation. The patient was 20-year-old male and had chest pain for 10 days. On simple chest x-ray and Thoracic CT scan, there was a large cystic mass surrounded with multiloculated round cysts with air fluid level on the right lower lobe of a lung. Right lower lobectomy was performed and the pathologic result was congenital cystic adenomatoid malformation.

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Results of Mitral Valve Repair in Patients with Congenital Mitral Disease (선천성 승모판막 기형 환자에서 승모판막 성형술)

  • Jang, Hee-Jin;Lee, Jeong-Ryul;Rho, Joon-Ryang;Kim, Yong-Jin;Kim, Woong-Han
    • Journal of Chest Surgery
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    • v.42 no.2
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    • pp.175-183
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    • 2009
  • Background: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement or pediatric mitral lesions can cause problems such as a lack of growth potential. There re only limited experiences with mitral valve repair at any institution, so the purpose of his study is to evaluate the outcomes of mitral valve repair n pediatric patients. Material and Method: Sixty-four consecutive children (28 males and 36 females) with a mean age of $5.5{\pm}4.7$ years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without trial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). Result: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there as no significant difference among the groups. The median follow-up was 4.6 years range: $0.5{\sim}12.2$ years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of $2.1{\pm}1.1$ procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there ere significant differences according to the repair strategies. Conclusion: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

Surgical Repair of Truncus Arteriosus in an Low-Birth Weight Premature Baby: Right Ventricular Outflow Reconstruction with Valveless Autologous Pericardial Conduit and the Result of 20-Month′s Follow-up (저체중조산아에서 동맥간의 교정 1예: 무판막 자가심낭도관의 이용과 20개월간의 추적결과)

  • 성시찬;양승인;이헝두;김시호;우종수;이영석
    • Journal of Chest Surgery
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    • v.36 no.10
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    • pp.766-771
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    • 2003
  • It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. Truncus arteriosus is a rare congenital anomaly that has an unfavorable natural course. We report a successful surgical correction of truncus arteriosus in an 13-day-old premature infant with body weight of 1.5 kg and gestational age of 32 weeks. We used autologous untreated pericardial conduit without valve in right ventricular outflow reconstruction. The patients remains in good condition with normal body weight (50 percentile) and wide right ventricular outflow tract 20 months after the operation.

Diffuse Supravalvar Aortic Stenosis Associated with Congenital Anomaly of the Aortic Valve(Williams Syndrome) -1 case report- (선천성 대동맥 판막 이상과 무명동맥 협착이 동반된 미만성 대동맥 판막상부 협착증 치험 1례)

  • 김수철;전순호
    • Journal of Chest Surgery
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    • v.33 no.9
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    • pp.748-751
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    • 2000
  • The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root(Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.

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Congenital Aortic Valvular Insufficiency Caused by Abnormal Valvular Structures in a Labrador Retriever Dog (래브라도 리트리버종 개의 비정상 판막 구조에 의한 선천성 대동맥 판막 부전)

  • Moon, Hyeong-Sun;Lee, Seung-Gon;Lee, Sang-Eun;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.24 no.2
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    • pp.233-237
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    • 2007
  • A 10-month-old intact male Labrador Retriever dog was referred with the primary complaint of exercise intolerance, especially after vigorous exercise. Physical examination revealed split S1 and grade III/VI diastolic regurgitant murmur at the left apex and base, respectively. ECG finding was normal sinus rhythm at rest, but supraventricular tachycardia with bundle branch blocks after exercise. Thoracic radiography revealed dilated ascending aorta with normal range of cardiac silhouette (VHS 10.2). Echocardiography revealed abnormal valvular structures just above the aortic valvular cusps causing aortic regurgitation with a reduction of left ventricular ejection fraction (LVEF). Based on those findings, the case was diagnosed as congenital aortic regurgitation caused by abnormal valvular structures. The dog was managed with diltiazem and exercise restriction. This is a rare case of aortic deformity in dogs.

Double Pulmonary Artery Sling with Multiorgan Abnormalities (다장기 기형을 동반한 이중 폐동맥 슬링)

  • Kang, Min-Woong;Na, Myung-Hoon;Lim, Seung-Pyung;Lee, Young;Kil, Hong-Ryang;Yu, Jae-Hyeon
    • Journal of Chest Surgery
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    • v.40 no.11
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    • pp.777-781
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    • 2007
  • Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery arises from the right pulmonary artery forming a sling around the trachea. This causes tracheal compression with the resulting respiratory symptoms. Most cases are associated with cardiovascular and tracheobronchial abnormalities. Some cases present incidentally without respiratory symptoms in adolescents and adults. We report a case with double left aberrant pulmonary artery associated with multiorgan anomalies which was incidentally found.

Congenital Cystic Adenomatoid Malformation Associated with Extralobar Pulmonary Sequestration -1 case report- (외엽형 폐격리증을 동반한 선천성 낭종성 선종양 기형 -1례 보고-)

  • Jeon, Sang-Hyeop;Kim, Byeong-Jun;Lee, Hyeong-Ryeol
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.223-226
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    • 1996
  • Pulmonary sequestration and congenital cystic adenomatoid malformation are two infrequent congenital pulmonary diseases and the combination of these two entities is rare. We had experienced a 3 week old male patient with a pulmonary mass, who had been sufyerring from tachypnea and chest wall retraction after birth. The pulmonary mass was suspected as a congenital cystic adenomat id malformation by chest CT. And therefore, we performed urgent operation via standard thoracotomy incision. Upon a thoracotomy, there was an extrapleural mass with anomalous blood supply near the posterior diaphragm and multiple cystic lesions in right lower lobe. The two anomalous arteries arising from the thoracic aorta and one vein draining into the azygos vein were ligated. and then the extrapleural mass was removed and a right lower lobectomy was performed. Final histologic diagnosis was congenital cystic adenomatoid malformation associated with extralobar pulmonary sequestration. The patient was discharged without any problem.

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